Cases reported "Cystic Fibrosis"

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1/41. Pitfall in the use of genotype analysis as the sole diagnostic criterion for cystic fibrosis.

    In this report, we present an asymptomatic infant, seen for a second opinion, who was given the diagnosis of cystic fibrosis (CF) as a neonate based on the presence of two mutant alleles, DeltaF508 and R117H. The diagnosis of CF adversely affected the family's emotional, employment, and financial statuses. Our evaluation included sweat chloride, nasal transepithelial potential difference, and bronchoscopy with bronchoalveolar lavage measurements, all which were consistent with findings expected from an individual without CF. genotype analysis for the sequence polymorphism in intron 8 of the cystic fibrosis transmembrane conductance regulator (CFTR) gene revealed the 7 thymidines and 9 thymidines alleles. We conclude that this patient probably expresses enough epithelial cell surface CFTR function such that she has a normal phenotype. Based on our evaluation, she does not meet the current diagnostic criteria for CF. Although genotype analysis can be an useful adjunct, it should not be the sole diagnostic criterion for CF.
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2/41. night blindness precipitated by isotretinoin in the setting of hypovitaminosis A.

    A 16-year-old male developed night blindness 2 weeks after starting isotretinoin at a dose of 20 mg per day for cystic acne. He also had cystic fibrosis, complicated by hepatic cirrhosis. Despite long-term oral vitamin a supplementation, serum vitamin a levels were found to be 0.3 mumol/L (normal range 0.9-2.5 mumol/L). Oral vitamin a replacement was instituted with resolution of his visual symptoms in 6 months. isotretinoin therapy was successfully continued with no deterioration in liver function. isotretinoin has been reported to cause deterioration in night vision. in vitro evidence suggests isotretinoin may interfere with the processing of endogenous vitamin a in the retina. This case highlights the need for careful monitoring of serum vitamin a status in patients with malabsorptive states on isotretinoin therapy.
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3/41. Renal failure and vestibular toxicity in an adolescent with cystic fibrosis receiving gentamicin and standard-dose ibuprofen.

    Gentamicin and standard-dose ibuprofen were administered to an adolescent with cystic fibrosis who developed renal failure and severe vestibulotoxicity. A contributing factor was possible suboptimal intravascular volume status. Because of the potential severity of this drug interaction, hydration status and renal and vestibular functions should be closely monitored in patients receiving ibuprofen and intravenous aminoglycosides concomitantly.
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4/41. Bronchoscopically administered recombinant human DNase for lobar atelectasis in cystic fibrosis.

    Lobar atelectasis is a common complication of cystic fibrosis. The majority of cases respond to intravenous antibiotics and chest physiotherapy. In a subgroup of patients, atelectasis is resistant to medical therapy, and its persistence in the pediatric population is associated with a poor prognosis. Bronchoscopic instillation of human recombinant DNase expanded atelectatic lobes in three children resistant to at least 2 weeks of medical therapy. This method of administration of DNase has been successful in resistant cases of lobar atelectasis in patients with chronic obstructive pulmonary disease, quadriplegia, and status asthmaticus. Purulent cystic fibrosis sputum has a very high dna content, and dna has been shown to become more pourable in vitro when treated with rhDNase. Bronchoscopic instillation of rhDNase should be considered in cases of persistent lobar atelectasis unresponsive to medical therapy.
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5/41. Cystic fibrosis in three children with bronchopulmonary dysplasia.

    Cystic fibrosis (CF) and bronchopulmonary dysplasia (BPD) are two common causes of chronic lung disease in children. patients with BPD or CF often have recurrent respiratory symptoms, failure to thrive, and/or metabolic alkalosis during infancy and childhood. Thus, recognizing the diagnosis of CF in an infant with BPD can be difficult. We present three infants with both BPD and CF. The infants shared a history of respiratory distress and prolonged oxygen requirements. All three also had difficulty gaining weight, even after pancreatic enzyme supplementation was instituted. Metabolic alkalosis was observed in two infants. Previous studies in children with CF suggest that early diagnosis may impact both lung health and nutritional status. A high index of suspicion is necessary for clinicians to identify these children early and intervene with appropriate therapy.
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6/41. riboflavin deficiency in cystic fibrosis: three case reports.

    Three cases of clinical riboflavin deficiency are reported in children aged 2-10 years attending a regional Cystic Fibrosis clinic. riboflavin deficiency presented as angular stomatitis in all three patients. patients were confirmed to be riboflavin deficient by assaying the activity of erythrocyte glutathione reductase. patients were not on routine supplements of water-soluble vitamins before presentation and were treated with riboflavin supplements as part of a water-soluble vitamin complex. At presentation, one patient had poor nutritional status, but two patients were adequately nourished, receiving overnight gastrostomy feeds. Data on these two patients indicate an adequate dietary intake of riboflavin, suggesting a mechanism for increased requirements, inadequate absorption or utilization. Additional deficiencies of thiamin, pyridoxine and iron were also observed. This paper reports the occurrence of a vitamin deficiency not previously reported in the cystic fibrosis population.
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7/41. hypersensitivity to inhaled TOBI following reaction to gentamicin.

    Cystic fibrosis (CF) is the most common autosomal-recessive disease in Caucasians. Colonization with pseudomonas aeruginosa (P. aeruginosa) of the CF airways causes deterioration of pulmonary status. TOBI (tobramycin solution for inhalation) is an inhaled antibiotic that can improve the pulmonary disease. We report on a 9-year old boy with CF who developed a rash following a course of IV gentamicin. The rash resolved after its discontinuation. However, the rash returned all over his body, with the start of inhalation of TOBI therapy. We desensitized the patient using escalating doses of inhaled TOBI. He tolerated the procedure well, and continues to be on TOBI 9 months after desensitization on a once-a-day regimen.
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8/41. Holistic approach of a child with cystic fibrosis: a case report.

    The pediatric dentistry Clinic staff of the Federal University of Santa Catarina (UFSC) treated a 5-year-old patient who had multiple dental treatment necessities. He also had cystic fibrosis (CF). As CF is an incurable disease that affects the mucous glands, especially the ones related to the lungs and digestive organs, the patient presented both chronic respiratory and intestinal problems. Such problems resulted in a severely compromised nutritional status. patients who have CF may present problems in oral health because they receive food supplementation that is rich in carbohydrates. Behavioral changes of such patients when in contact with health professionals are also a point to consider because these changes may make the treatment difficult. That is the reason why oral complications imposed by the disease, as well as the psychological aspects related to it, need to be discussed so that treatment is given to the patient aiming at physical and emotional comfort.
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9/41. Hemolytic anemia and edema as the initial signs in infants with cystic fibrosis. Consider this diagnosis even in absence of pulmonary symptoms.

    This is a report of five infants eventually proven to have cystic fibrosis of the pancreas, who presented with hemolytic anemia and edema. Since the sweat test is often unreliable in edematous states, the possibility of cystic fibrosis should be considered. Use of either a protein hydrolysate or addition of pancreatic enzymes will improve nutritional status, and when edema disappears, a definite sweat test can be performed. Evidence of vitamin e deficiency in some of the cases is presented, and the possibility of this as a cause of anemia is raised.
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10/41. Biopsychosocial issues and risk factors in the family when the child has a chronic illness.

    Between 10 and 20 million American children and adolescents have some type of chronic health condition or impairment. There has been a recent interest in the psychological aspects--emotional and behavioral--of chronic illness in children and adolescents. Major new areas of science, such as behavioral medicine, have emerged to address the research and clinical demands of this field. Several studies already have demonstrated the increased risk for psychiatric problems in children with chronic health conditions to be three to four times greater than their healthy peers. The proposed perspective shifts from a traditional sole reliance on medical diagnosis to a focus more on the dimensions of adjustment, socioeconomic status, visibility of condition, social support, and family functioning.
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